N-acetyl-L-cysteine

Three steps

• N-(9Z-octadecenoyl)glycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (9Z)-octadecenoyl-CoA + glycine = N-(9Z-octadecenoyl)glycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 2 moremitochondrial matrix, extracellular space, cytosol

• N-(9Z-octadecenoyl)-L-phenylalanineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. L-phenylalanine + (9Z)-octadecenoate = N-(9Z-octadecenoyl)-L-phenylalanine + H2O

  Catalysed by NAGS, SLC7A5, PM20D1mitochondrial matrix, extracellular space, cytosol

• N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. (5Z,8Z,11Z,14Z)-eicosatetraenoate + L-phenylalanine = N-(5Z,8Z,11Z,14Z-eicosatetraenoyl)-L-phenylalanine + H2O

  Catalysed by NAGS, SLC7A5, PM20D1mitochondrial matrix, extracellular space, cytosol

• N-(9Z-octadecenoyl)-L-leucineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and leucine (cytosol to extracellular)
  3. N-(9Z-octadecenoyl)-L-leucine + H2O = L-leucine + (9Z)-octadecenoate

  Catalysed by NAGS, SLC7A9, SLC3A1, PM20D1mitochondrial matrix, extracellular space, cytosol

• N-octadecanoylglycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. octadecanoyl-CoA + glycine = N-octadecanoylglycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• N-(9Z-hexadecenoyl)-glycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (9Z)-hexadecenoyl-CoA + glycine = N-(9Z-hexadecenoyl)-glycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• acetoacetateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. transport of acetoacetate and pyruvate (cytosol to mitochondria)

  Catalysed by NAGS, CTHmitochondrial matrix, cytosolOne of 4 routes

• 3-sulfinopyruvateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-Cysteine:oxygen oxidoreductase
  3. 3-sulfino-L-alanine:2-oxoglutarate aminotransferase

  Catalysed by NAGS, CDO1, GOT1mitochondrial matrix, cytosolOne of 2 routes

• N-hexadecanoylglycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. hexadecanoyl-CoA + glycine = N-hexadecanoylglycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• N-dodecanoylglycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. dodecanoyl-CoA + glycine = N-dodecanoylglycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• N-decanoylglycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. decanoyl-CoA + glycine = N-decanoylglycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• N-octanoylglycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. octanoyl-CoA + glycine = N-octanoylglycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• D-histidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and ornithine (cytosol to extracellular)
  3. D-histidine(in) + L-ornithine(out) + H(+)(in) = D-histidine(out) + L-ornithine(in) + H(+)(out)

  Catalysed by NAGS, SLC7A6, SLC25A2mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 2 routes

• L-homoarginineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of arginine and cysteine (cytosol to extracellular)
  3. L-homoarginine(in) + L-arginine(out) = L-homoarginine(out) + L-arginine(in)

  Catalysed by NAGS, SLC7A6, SLC25A29mitochondrial matrix, extracellular space, cytosol, inner side of the membrane, outer side of the membraneOne of 4 routes

• L-homocarnosineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and histidine (cytosol to extracellular)
  3. Formation of homocarnosine

  Catalysed by NAGS, SLC7A6, CARNS1mitochondrial matrix, extracellular space, cytosol

• N(1)-(5-phospho-beta-D-ribosyl)glycinamideReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. 5-phospho-beta-D-ribosylamine + glycine + ATP = N(1)-(5-phospho-beta-D-ribosyl)glycinamide + ADP + phosphate + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• L-cystine-bis-glycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. thioredoxin-disulfide reductase (NADPH) (cystine)
  3. L-cystine-bis-glycine + 2 H2O = L-cystine + 2 glycine

  Catalysed by NAGS, TXNRD1, TXNRD3, TXNRD2 and 2 moremitochondrial matrix, cytosolOne of 2 routes

• glycyldehydrophenylalanineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. glycyldehydrophenylalanine + H2O = 2,3-didehydrophenylalanine + glycine

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glutamine (cytosol to extracellular)
  3. N(2)-formyl-N(1)-(5-phospho-beta-D-ribosyl)glycinamide + L-glutamine + ATP + H2O = 2-formamido-N(1)-(5-O-phospho-beta-D-ribosyl)acetamidine + L-glutamate + ADP + phosphate + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A10 and 1 moremitochondrial matrix, extracellular space, cytosol

• kinetensinReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and phenylalanine (cytosol to extracellular)
  3. Chymase

  Catalysed by NAGS, SLC7A5, CMA1mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• an N-(fatty acyl)-glycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. a fatty acyl-CoA + glycine = an N-(fatty acyl)-glycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• N-(9Z,12Z-octadecadienoyl)-glycineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. (9Z,12Z)-octadecadienoyl-CoA + glycine = N-(9Z,12Z-octadecadienoyl)-glycine + CoA + H(+)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• (7Z)-octadecenoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. carnitine O-palmitoyltransferase ((7Z)-octadecenoyl-CoA)

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 3 routes

• acetaldehydeReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and threonine (cytosol to extracellular)
  3. L-threonine acetaldehyde-lyase (glycine-forming)

  Catalysed by NAGS, SLC7A5mitochondrial matrix, extracellular space, cytosol

• CHEBI:15345Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Acetoacetate:CoA ligase (AMP-forming)

  Catalysed by NAGS, AACSmitochondrial matrix, cytosolOne of 2 routes

• cholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Acetyl-CoA:choline O-acetyltransferase

  Catalysed by NAGS, CHATmitochondrial matrix, nucleusOne of 2 routes

• acetylcholineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. cystine to cysteine conversion
  3. transport of acetylcholine and GSH (cytosol to extracellular)

  Catalysed by NAGS, SLCO1A2, SLCO1B3mitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• acetylpyruvateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. acetylpyruvate + H2O = acetate + pyruvate + H(+)

  Catalysed by NAGS, CTH, FAHD1mitochondrial matrix, cytosol

• all-trans-retinoic acidReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. transport of pyruvate and retinoate (cytosol to extracellular)

  Catalysed by NAGS, CTH, SLC16A4, SLC16A5 and 4 moremitochondrial matrix, cytosol, extracellular spaceOne of 2 routes

• 4a-hydroxytetrahydrobiopterinReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. L-tryptophan,tetrahydrobiopterin:oxygen oxidoreductase(5-hydroxylating)

  Catalysed by NAGS, SLC7A5, TPH1, TPH2mitochondrial matrix, extracellular space, cytosolOne of 3 routes

• CHEBI:15380Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. 5-aminolevulinate synthase (2-amino-3-oxoadipate)

  Catalysed by NAGS, ALAS1, CPNE7mitochondrial matrixOne of 8 routes

• CHEBI:15477Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. glycine N-acyltransferase (2-methylbutyryl-CoA)

  Catalysed by NAGS, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 2 routes

• 2-methylcrotonoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Production of Tiglylcarnitine

  Catalysed by NAGS, CPT1A, CPT1Bmitochondrial matrix, cytosolOne of 2 routes

• isobutyryl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. acetyl-CoA C-acyltransferase (3-oxo-4-methyl-pentanoyl-CoA)

  Catalysed by NAGS, HADHA, ACAA2, HADHBmitochondrial matrixOne of 3 routes

• 3-methylbut-2-enoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. glycine-N-acyltransferase (3-methylcrotonoylglycine)

  Catalysed by NAGS, GLYATmitochondrial matrix

• isovaleryl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. glycine N-acyltransferase (isovalerylglycine)

  Catalysed by NAGS, GLYAT, GLYATL2, GLYATL1mitochondrial matrixOne of 3 routes

• arachidonoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Arachidonate:CoA ligase (AMP-forming)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

• CHEBI:15519Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. propanoyl-CoA C-acyltransferase

  Catalysed by NAGS, ACAA1, SCP2, MYO5Bmitochondrial matrix, peroxisomeOne of 2 routes

• palmitoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Palmitate:CoA ligase (AMP-forming)

  Catalysed by NAGS, SLC27A2, ACSL4, ACSL3 and 5 moremitochondrial matrix, cytosolOne of 11 routes

• oleoyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Transport of octadecenoyl-CoA into Mitochondrial Matrix

  Catalysed by NAGS, CPT1A, CPT1C, CPT1Bmitochondrial matrix, cytosolOne of 11 routes

• phenylacetyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Phenylacetate:CoA ligase

  Catalysed by NAGSmitochondrial matrix, cytosolOne of 3 routes

• propionyl-CoAReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. Propanoate:CoA ligase (AMP-forming)

  Catalysed by NAGS, ACSS3, ACSS2, ACSS1mitochondrial matrix, cytosolOne of 43 routes

• CHEBI:15570Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. transport of D-alanine and glycine (cytosol to extracellular)

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosolOne of 5 routes

• (S)-malateReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. L-cysteine hydrogen-sulfide-lyase (deaminating
  3. (S)-malate + NAD(+) = pyruvate + CO2 + NADH

  Catalysed by NAGS, CTH, ME2mitochondrial matrix, cytosolOne of 2 routes

• CHEBI:15611Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and glycine (cytosol to extracellular)
  3. S-Adenosyl-L-methionine:glycine N-methyltransferase

  Catalysed by NAGS, SLC3A2, SLC7A5, SLC7A8 and 1 moremitochondrial matrix, extracellular space, cytosol

• (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterinReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and tryptophan (cytosol to extracellular)
  3. (6R)-L-erythro-5,6,7,8-tetrahydrobiopterin + L-tryptophan + O2 = 5-hydroxy-L-tryptophan + (4aS,6R)-4a-hydroxy-L-erythro-5,6,7,8-tetrahydrobiopterin

  Catalysed by NAGS, SLC7A5, TPH1, TPH2mitochondrial matrix, extracellular space, cytosolOne of 2 routes

• CHEBI:15650Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N-acetyl-LTE4 to leukotriene E4 conversion

  Catalysed by NAGS, ZDHHC11B, NAA10, ESCO2 and 29 moremitochondrial matrix, cytosolOne of 4 routes

• betaine aldehydeReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. Mo-molybdopterin + L-cysteine + AH2 = thio-Mo-molybdopterin + L-alanine + A + H2O
  3. choline + A = betaine aldehyde + AH2

  Catalysed by NAGS, MOCOS, CHDHmitochondrial matrix

• trimethylenediamineReach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. transport of cysteine and lysine (cytosol to extracellular)
  3. deoxyhypusine synthase (1,3-diaminopropane)

  Catalysed by NAGS, SLC7A6, DHPSmitochondrial matrix, extracellular space, cytosol

• CHEBI:15727Reach from here →
  1. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  2. N-acetylglutamate synthase (N-acetyl-L-cysteine)
  3. N-alpha-acetyltransferase 10, NatA catalytic subunit (acetylcarnosine)

  Catalysed by NAGS, NAA10mitochondrial matrix, cytosolOne of 2 routes